Mouse Genome Informatics
hm
    Gp1bbtm1Frla/Gp1bbtm1Frla
involves: 129S2/SvPas * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
hematopoietic system
• discoid platelets have enlarged diameter compared to wild-type
• platelets display 2-fold increase in number of microtubules
• platelet counts are reduced by 73%

homeostasis/metabolism
• 76% of mice bled for more than 20 minutes compared to 82% of wild-type having bleeding times of <12 minutes
• platelet adhesion from blood at high shear forces (3000 s-1) is nearly abolished (98% decrease) relative to wild-type blood; platelet adhesion and aggregation is observed at greatly reduced size compared to wild-type at a shear rate of 1500 s-1
• in vivo, application of FeCl3 produces stable occlusion in 12.5% of arteries, while 25% show unstable occlusions and 62.5% do not occlude after 30 minutes compared to 75% in wild-type; thrombi are less extensive than wild-type with little sign of degranulation
• laser beam-induced superficial lesions of mesenteric arterioles results in almost no thrombus formation compared to wild-type while thrombi after severe lesions are reduced to only 25% of wild-type at 160 seconds

Mouse Models of Human Disease
OMIM IDRef(s)
Bernard-Soulier Syndrome; BSS 231200 J:135065